Cystic Fibrosis (Condition)

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited disease characterized by an abnormality in the glands that produce sweat and mucus. Due to improved treatments, people with CF, on average, live into their mid to late 30s.

Cystic fibrosis affects various systems in children and young adults, including the following:

• respiratory system
• digestive system
• reproductive system

There are about 30,000 people in the US who are affected with the disease, and about 1,000 babies are diagnosed with it each year. It occurs mainly in Caucasians, who have a northern European heredity, although it also occurs in African-Americans, Asian Americans, and Native Americans.

Approximately one in 31 people in the US are carriers of the cystic fibrosis gene. These people are not affected by the disease and usually do not know that they are carriers.

How does CF affect the gastrointestinal (GI) system?

The organ primarily affected is the pancreas, which secretes substances that aid digestion and help control blood-glucose levels.

As a result of the abnormal electrolyte transport system in the cells, the secretions from the pancreas become thick and lead to an obstruction of the ducts of the pancreas. This obstruction then causes a decrease in the secretion of enzymes from the pancreas that normally help to digest food. A person with CF has difficulty absorbing proteins, fats, and vitamins A, D, E, and K.

The problems with the pancreas can become so severe that some of the cells in the pancreas can become destroyed. This may lead to glucose intolerance and insulin-dependent diabetes. About 35 percent of CF patients develop this type of diabetes in their 20s and more than 40 percent develop the disease after 30 years of age.

The symptoms that may be present due to the involvement with the GI tract include the following:

• bulky, greasy stools
• rectal prolapse – a condition in which the end part of the bowels comes out of the anus.
• delayed puberty
• fat in the stools
• stomach pain
• bloody diarrhea

The liver may also be affected. A small number of patients may actually develop liver disease. Symptoms of liver disease may include:

• enlarged liver
• swollen abdomen
• yellow color to the skin
• vomiting of blood

Treatment for cystic fibrosis:

Specific treatment for cystic fibrosis will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• expectations for the course of the disease
• your tolerance for specific medications, procedures, or therapies
• your opinion or preference